Onco Wiki / drapeau rouge

Néoplasme myéloproliferatif (polycythémie vera [PV], thrombocythmie essentielle [ET], ou p...

Vue déterministe de l'entité source de la LJAM. L'autorité clinique demeure avec les ID de la source cités et l'état d'approbation de l ́examinateur.

IDRF-MPN-SECONDARY-LEUKEMIA-PREVENTION
TypeSignal d’alerte
Étatreviewed 2026-05-18 | pending_clinical_signoff
MaladiesDIS-AML
SourcesSRC-NCCN-AML-2025 SRC-NCCN-BCELL-2025 SRC-NCCN-MPN-2025

Origine du drapeau rouge

DéfinitionMyeloproliferative neoplasm (polycythemia vera [PV], essential thrombocythemia [ET], or primary myelofibrosis [PMF]) per WHO 2022 criteria, OR a clinical phenotype suggestive of MPN (driver-mutation positivity — JAK2 V617F, JAK2 exon 12, CALR exon 9, or MPL W515 — with sustained myeloproliferative blood-count abnormality but not yet meeting full WHO criteria) in an individual without a current diagnosis of acute myeloid leukemia (AML) or post-MPN myelofibrosis. MPN clones carry inherent risk of clonal evolution to higher-grade myeloid neoplasia: cumulative risk of leukemic transformation (sAML / blast-phase MPN) ~5% in PV, ~3% in ET, ~10-20% in PMF at 10-15 years; cumulative risk of progression from PV/ET to post-PV/ post-ET myelofibrosis ~10-15% at 15 years. Standard MPN management reduces thrombosis (the dominant short-term mortality driver) but does not abolish leukemic-transformatio...
Direction cliniqueenquêter
Catégorieautres

Logique de déclenchement

{
  "any_of": [
    {
      "finding": "jak2_v617f_positive_without_confirmed_mpn_disease",
      "value": true
    },
    {
      "finding": "calr_exon9_mutation_positive_without_confirmed_mpn_disease",
      "value": true
    },
    {
      "finding": "mpl_w515_mutation_positive_without_confirmed_mpn_disease",
      "value": true
    },
    {
      "finding": "mpn_phenotype_pre_who_criteria",
      "value": true
    }
  ],
  "type": "lab_value"
}

Notes

v0.3 chronic-condition prevention pilot — MPN / secondary AML prevention. Prevention-persona RedFlag (CHARTER §3 amended 2026-05-18 Path A, HCP-mediated). Fires when patient profile shows MPN-driver- mutation positivity (JAK2 V617F, CALR exon 9, MPL W515) WITHOUT a confirmed MPN Disease entity (DIS-PV, DIS-ET, DIS-PMF) AND no confirmed AML or post-MPN MF. Engine routes to PreventionPlan with 2 tracks: CBC + smear q3-6mo + bone marrow on cytopenia/blast emergence + JAK2/CALR/MPL allele-burden monitoring (IND-MPN-SECONDARY-LEUKEMIA-PREVENTION-SURVEILLANCE) as standard; hematology-driven observation with bone marrow only on cytopenia trigger (IND-MPN-SECONDARY-LEUKEMIA-PREVENTION-OBSERVATION) as alternative. Engine design note: patients with confirmed DIS-PV / DIS-ET / DIS-PMF are routed via the treatment-persona MPN management algorithm (cytoreduction with hydroxyurea or ruxolitinib, aspirin for thrombosis prevention, phlebotomy for PV, etc.) and do NOT enter the §20 prevention path. This RF therefore covers the narrow set of driver-mutation-positive patients in pre-MPN-criteria phase or those whose MPN status is still under workup but who carry molecular evidence of clonal myelopro...

Utilisé par

Indication